Glanzmann's thrombasthenia

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Glanzmann's thrombasthenia

Hematological system

Coagulation disorders

Hemophilia

Vitamin K deficiency

Leukemoid reaction

Leukemoid reaction

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Glanzmann's thrombasthenia

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Glanzmann's thrombasthenia

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USMLE® Step 1 style questions USMLE

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A 9-year-old boy is brought to the emergency department by his parents due to prolonged bleeding following a tooth extraction earlier in the day. Past medical history is noncontributory. Temperature is 37.5°C (99.5°F), pulse is 88/min, respirations are 14/min, and blood pressure is 112/62 mmHg. Physical exam shows gingival bleeding and petechiae. Laboratory testing is obtained, and the results are shown below.  
 
Laboratory value  Result
 Hematologic  
 Hemoglobin  12 g/dL 
 Hematocrit  40% 
 Platelet count  95,000/mm3  
 Leukocyte count  9,000/mm3  
Coagulation studies  
 Prothrombin time (PT)  12 seconds 
 Activated partial thromboplastin time (aPTT)  29 seconds 
 Bleeding time*  15 minutes 
*Reference Range: 2-7 minutes  

Which of the following conditions is the patient at greatest risk of developing?   

External References

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Glanzmann thrombasthenia p. 419, 434

Summary

Glanzmann's thrombasthenia is a rare autosomal recessive bleeding disorder caused by defects in platelet aggregation. In Glanzmann's thrombasthenia, platelets are deficient in the fibrinogen receptor GpIIb/IIIa necessary for proper platelet aggregation. This leads to easy bruising, mucosa bleeding, and prolonged bleeding from even minor cuts. It can also cause internal bleeding, which can be life-threatening.

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